em Ann Trop Paediatr /em 2007; 27:81C86

em Ann Trop Paediatr /em 2007; 27:81C86. on steroids, albumin, and high molecular pounds plasma expanders (hydroxyethyl starch). Because of high hematocrit, phlebotomy was also performed. The patient had complete clinical remission and a diagnosis of SCLS was finally made. He received prophylactic therapy with verapamil and theophylline that was self-stopped for intolerance (hypotension and tachycardia). He had a new crisis 2 days after a physical effort, and was admitted in intensive care unit. The patient died for severe hypovolemic shock with multiorgan failure and sudden cardiac arrest 15 hours after hospital admission. Postmortem investigation revealed massive interstitial edema of main organs with myocardial hyperacute ischemia. Studies on SCLS are limited for the rarity of the disease and its unpredictable course. Both prophylactic and acute crisis treatments are empirical and optimal management of severe attacks is still lacking. INTRODUCTION Systemic capillary leak syndrome (SCLS), also known as Clarkson disease, is a rare disorder characterized by episodes of severe hypotension, hypoalbuminemia, and hemoconcentration.1 A monoclonal gammopathy of unknown significance, typically of the immunoglobulins of class G (IgG) class, is present in most of the SCLS adult cases.2,3 During acute crisis of SCLS, profound derangement of the vascular endothelium results in leakage of plasma and proteins into the interstitial compartment. Episodes differ in severity and frequency and may be life-threatening. SCLS was first described about 50 years ago, and is variably referred to as Clarkson disease or syndrome. 1 Approximately 250 cases of SCLS have been reported worldwide since 1960.3C5 The 5-year survival rate is about 75%, and deaths are most commonly related to acute SCLS events. These have been diagnosed primarily in middle-aged adults, although cases in children have also been reported.5,6 We describe here a case of fatal SCLS associated with physical effort. CASE PRESENTATION A 49-year-old Italian man was admitted to hospital in August 2012 for suspected gastroenteritis. He was a pharmaceutical representative practicing sports during free time. The patient had a previous diagnosis of microurolithiasis with calcific prostatitis, and his relevant clinical history had began in April 2012, 2 days after a marathon (a long-distance running with of 42?km) with a flu-like syndrome characterized by fever (37.5C), muscle weakness, and dyspnoea. On that occasion, he went to the emergency department (ED) where his blood tests revealed high hemoglobin levels (18.9?g/dL, hematocrit 54%), with mild increase in serum creatinine (1.3?mg/dL). Symptoms and blood test were ascribed to dehydratation secondary to the prolonged physical effort. Assumption of eritropoietin as anabolic substance and/or diuretics was also suspected but firmly denied by the patient. He therefore underwent hydratation and phlebotomy. He was discharged and then addressed to hematological investigation. Primary or secondary polycythemia was excluded by the absence of mutation in Janus kinase 2 gene and normal arterial gas analysis. Clinically, the patient rapidly improved but in May 2012 he developed progressive swelling of the face and of the lower limbs within 12 hours, Riluzole (Rilutek) after apparently no preceding efforts, with Riluzole (Rilutek) body weight increase of about 10?kg. Oral furosemide (50?mg) and steroid (prednisone 25?mg) assumption given by his general practitioner for a few days was followed by prompt reduction of edema and body weight. On 23 August 2012, he had Riluzole (Rilutek) a running of 18?km. Three days later, he complained of gastrointestinal symptoms with nausea, vomiting, abdominal pain, diarrhea, and fever (38.5C). He therefore took oral paracetamol 500? mg thrice a day and metoclopramide for 2 days with no appreciable improvement. He went to the ED for the occurrence of deep asthenia, hypotension, and oligoanuria. Blood test showed hemoglobin levels of 22?g/dL, hematocrit 64%, and creatinine 2.7?mg/dL (Table ?(Table1).1). The patient was therefore admitted to the Internal Medicine ward. He presented with bilateral edema of the legs and forearms. The blood pressure was 90/50?mm Hg, the electrocardiogram (ECG) showed sinus tachycardia. Echocardiography and thoracoabdominal computed tomography ITGA8 were negligible. Hypotension was treated with saline solutions and dopamine infusion (4?mcg/kg/min). However, because of persistent hypotension and occurrence of increasing anasarcatic state with lower limb weakness, he was transferred to intensive care unit where he was treated with.